Light chain amyloidosis (AL amyloidosis) is a rare plasma cell dyscrasia characterized by the deposition of insoluble amyloid fibrils in multiple organ systems. The treatment of amyloidosis primarily relies on anti-plasma cell therapy and supportive care. The application of anti-plasma cell therapy has significantly improved outcomes in patients with AL amyloidosis. Standard first-line therapy typically includes daratumumab, bortezomib, cyclophosphamide, and dexamethasone (Dara-BCD), achieving a complete hematologic response in nearly 60% of patients.The depth and speed of the hematologic response are strongly correlated with organ response and overall survival. An early achievement of a complete hematologic response is particularly crucial in cases of AL amyloidosis characterized by significant organ involvement. The median time to a hematologic response for the daratumumab based treatment is only 7-9 days. The retrospective data showed that the hematologic response in Day 7 in Cycle 1 (C1D7) may predict the complete hematologic response rate. In order to validate the conclusion, the investigator design this prospective study.
Study Type
OBSERVATIONAL
Enrollment
50
The treament follow the clinical routine practice, as daratumumab 16mg/kg iv qw OR dara SC 1800mg initially. The treatment schedule involve weekly administrations during cycles 1 to 2, following by biweekly administrations during cycles 3 to 6. Subsequently, monthly administrations are given as monotherapy.
Bortezomib is administered subcutaneously. The dosage range from 0.7 to 1.3 mg/m2 on days 1, 8, 15, and 22 of each cycle, for a maximum of 6 cycles
Dexamethasone is administered intravenously at a dose of 40 mg weekly for each cycle, for a maximum of 6 cycles. For patients over 70 years of age or with severe edema, heart failure, or uncontrolled diabetes mellitus, dexamethasone can be administered at a dose of 10-20 mg per week.
Some patients may receive cyclophosphamide orally or intravenously at a dosage of 300 mg/m2 weekly.
The treament follow the clinical routine practice, as daratumumab 16mg/kg iv qw OR dara SC 1800mg initially. The treatment schedule involve weekly administrations during cycles 1 to 2, following by biweekly administrations during cycles 3 to 6. Subsequently, monthly administrations are given as monotherapy.
Peking University People's Hospital
Beijing, Beijing Municipality, China
RECRUITINGFuxing Hospital affiliated to Capital Medical University
Beijing, Beijing Municipality, China
RECRUITINGBeijing Anzhen Hospital
Beijing, Beijing Municipality, China
RECRUITINGPeking University First Hospital
Beijing, Beijing Municipality, China
RECRUITINGFirst Affiliated Hospital of Harbin Medical University
Harbin, Heilongjiang, China
RECRUITINGChinese PLA Eastern Theater General Hospital
Nanjing, Jiangsu, China
RECRUITINGShengjing Hospital of China Medical University
Shenyang, Liaoning, China
RECRUITINGThe First Affiliated Hospital of Xi'an Jiao Tong University
Xi'an, China
RECRUITINGComplete hematologic response
Time frame: 6 months
Overall hematologic response
Time frame: 6 months
At least one organ response
Time frame: 12 months
Minimal residual disease
Bone marrow miminal residual disease
Time frame: 6 months, 12 months
TTNT
Time to next treatment
Time frame: 12 months
MOD-EFS
Major Organ Deterioration Event-Free Survival (MOD-EFS) is defined as the time from the beginning of treatment to hematologic progression, clinical manifestation of end-stage cardiac or renal disease, initiation of subsequent other anti-plasma cell therapy, or death, whichever occur first
Time frame: 12 months
MOD-PFS
Major organ deterioration progression-free survival (MOD-PFS) is defined as the time from the beginning of treatment to death, clinical manifestation of end-stage cardiac or renal failure, or hematologic progression, whichever occur first
Time frame: 12 months
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